Improving the Experience of Using Non-invasive Ventilation in Children

Abstract

Background: Advancement in technology and a push to move long-term care out of hospital has led to an increased use of long-term non-invasive ventilation (LT-NIV) in children with sleep-related breathing disorders or respiratory insufficiency. NIV indications have expanded to include children with various complex medical conditions including children with upper airway obstruction, neuromuscular disorders, cardiorespiratory problems, and central nervous system (CNS) disorders. Children who are dependent on NIV technology have technical and practical burdens that affect daily life, sleep and family routines. Evidence of the benefits of NIV for children is variable with limited data for children with CNS disorders and varied outcome reporting across studies. Families also report challenges with equipment, mask fit, and limited involvement in decisions about care and research. These gaps restrict progress toward child and family-centred care for children using LT-NIV. Aim: This thesis focuses on understanding the experience of using LT-NIV for children, including summarizing the evidence for LT-NIV use in children with CNS disorders. The goal is to identify clinical outcomes related to LT-NIV use, understand child and family priorities with respect to clinical care and participation in research related to LT-NIV use, and outline strategies to improve the experience of NIV for children. Methods: This thesis includes two studies using different methods. The first study is a systematic review that evaluated outcomes related to LT-NIV use in children with CNS disorders. Following systematic review and meta-analysis guidelines, articles reporting on hospitalization, respiratory outcomes, sleep parameters, quality of life, and adherence in children with central nervous system disorders using long term NIV were identified and summarized. The second study is a mixed methods study that used a survey to collect information from parents/guardians of children using LT-NIV. The survey questions related to how children using LT-NIV and their families could be involved in the research process to improve the experience of using LT-NIV, and what outcomes related to LT-NIV use were most important to children and their families. To describe the participants and their child who used LT-NIV, socio-demographic and medical chart data related to LT-NIV use were also collected. Results: The systematic review demonstrated that children with CNS using LT-NIV are a diverse group of children who make up a sizeable proportion of children using LT-NIV. Outcome reporting across studies was limited and inconsistent. Mortality did not differ between children with CNS disorders and other children using LT-NIV. Hospitalization rates may be higher in children with CNS disorders using LT-NIV compared to children without CNS disorders. LT-NIV improves sleep and respiratory events in children with CNS disorders. The mixed-methods study findings showed that children using LT-NIV and their parents are experts in LT-NIV use and highly motivated to be part of the research process. Parents/guardians are motivated by a desire to advance knowledge and understanding of NIV technology, wanting to share their knowledge to help other families to be successful using LT-NIV as well as improving their own experience. Children taking part in research may motivate them to improve their LT-NV use. Outcomes of interest most important to parents/guardians were not the clinical outcomes measured in NIV clinic but rather outcomes related to a better nights rest and better daytime function. Conclusion: LT-NIV offers benefits for some children with CNS disorders, but evidence on which children will benefit from this technology is limited. Families’ experiences highlight priorities that differ from those used in clinical decision making. The combined findings support shifting research and clinical care toward approaches that integrate children and families as partners in LT-NIV research and emphasize practical, meaningful outcomes as measures of LT-NIV success.